Leber's hereditary optic neuropathy

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Related to Leber's optic atrophy: Leber disease, aphakia, achromatopsia, LHON

Leber's hereditary optic neuropathy

A rare genetic disease inherited from the mother caused by a point mutation in MITOCHONDRIAL DNA. Onset is usually about the 20th year and males are affected more often than females. It features changes in the small retinal blood vessels progressing to a degree of atrophy of the optic nerves in both eyes with a severe defect in visual acuity. In most cases a visual acuity of about 6/60-one tenth of normal-is retained. (T. von Leber, 1040–1917, German ophthalmologist and professor of ophthalmology at Heidelberg).
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
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